Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.Retroperitoneal fibrosis can be of 2 types...Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.Retroperitoneal fibrosis can be of 2 types:idiopathic and secondary.The recently advocated concept and diagnostic criteria of immunoglobulin G4(IgG4)-related disease,derived from research on autoimmune pancreatitis(AIP),has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease.We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease;however,the actual prevalence is unclear.Conversely,some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease.Because retroperitoneal fibrosis has no specific symptoms,diagnosis is primarily based on diagnostic imaging(computed tomography and magnetic resonance imaging),which is also useful in evaluating the effect of therapy.Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP.Thus,the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis.High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease.The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause.For patients with concurrent AIP,i.e.,IgG4-related retroperitoneal fibrosis,the starting dose of steroid is usually 30-40 mg/d.The response to steroid therapy is generally favorable.In most cases,the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment.However,the epidemiology,treatment for recurring retroperitoneal fibrosis,and long-term prognosis are still largely unknown.Further analysis of such cases and research are necessary.展开更多
This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves ...This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.展开更多
目的总结分析IgG4相关性疾病(immunoglobulin-G4 related disease,IgG4-RD)的妊娠及产后临床资料、诊治过程及随访结果,以提高产科医生对IgG4-RD的认识水平。方法采用回顾性研究方法对2023年1月18日北京大学深圳医院收治的1例(本例患者)...目的总结分析IgG4相关性疾病(immunoglobulin-G4 related disease,IgG4-RD)的妊娠及产后临床资料、诊治过程及随访结果,以提高产科医生对IgG4-RD的认识水平。方法采用回顾性研究方法对2023年1月18日北京大学深圳医院收治的1例(本例患者)IgG4相关甲状腺疾病成功妊娠的诊疗经过,以及通过数据库检索与妊娠相关的IgG4-RD病例报道进行分析。结果本例患者29岁,发现甲亢4年余、IgG4-RD 7个月、双胎妊娠,孕29周时因"甲亢危象、心功能Ⅳ级、IgG4-RD、子痫前期、新型冠状病毒感染等"于我院经多学科共同诊治,获得良好围产结局。文献检索到IgG4-RD合并妊娠病例报道6篇(共5例患者),包括本例患者在内的6例患者受累器官、临床表现不尽相同,经激素规范治疗后均获得良好围产结局。结论妊娠相关的IgG4-RD表现复杂多样,严重者可致重要脏器功能障碍。IgG4相关甲状腺疾病女性能够成功妊娠但存在较大风险,孕期及产后需持续激素维持治疗、多学科严密监测。展开更多
基金Supported by The Research Program of Intractable Disease and the Research Committee of Intractable Pancreatic Diseases of the Ministry of Health,Labor and Welfare of Japan
文摘Retroperitoneal fibrosis is a rare disease characterized by the development of inflammation and fibrosis in the soft tissues of the retroperitoneum and other abdominal organs.Retroperitoneal fibrosis can be of 2 types:idiopathic and secondary.The recently advocated concept and diagnostic criteria of immunoglobulin G4(IgG4)-related disease,derived from research on autoimmune pancreatitis(AIP),has led to widespread recognition of retroperitoneal fibrosis as a condition caused by IgG4-related disease.We now know that previously diagnosed idiopathic retroperitoneal fibrosis includes IgG4-related disease;however,the actual prevalence is unclear.Conversely,some reports on AIP suggest that retroperitoneal fibrosis is concurrently found in about 10% of IgG4-related disease.Because retroperitoneal fibrosis has no specific symptoms,diagnosis is primarily based on diagnostic imaging(computed tomography and magnetic resonance imaging),which is also useful in evaluating the effect of therapy.Idiopathic retroperitoneal fibrosis can occur at different times with other lesions of IgG4-related disease including AIP.Thus,the IgG4 assay is recommended to diagnose idiopathic retroperitoneal fibrosis.High serum IgG4 levels should be treated and monitored as a symptom of IgG4-related disease.The first line of treatment for retroperitoneal fibrosis is steroid therapy regardless of its cause.For patients with concurrent AIP,i.e.,IgG4-related retroperitoneal fibrosis,the starting dose of steroid is usually 30-40 mg/d.The response to steroid therapy is generally favorable.In most cases,the pancreatic lesion and retroperitoneal fibrosis improve after the initial treatment.However,the epidemiology,treatment for recurring retroperitoneal fibrosis,and long-term prognosis are still largely unknown.Further analysis of such cases and research are necessary.
文摘This review will encompass definition, pathogenesis, renal clinical manifestations and treatment of immunoglobulin G4-related diseases( IgG4-RDs). IgG4-RD is a recently recognized clinical entity that often involves multiple organs and is characterized by high levels of serum immunoglobulins G4, dense infiltration of IgG4+ cells and storiform fibrosis. Cellular immunity, particularly T-cell mediated immunity, has been implicated in the pathogenesis of IgG4-RDs. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulopathy and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis. IgG4-RD diagnosis should be based on specific histopathological findings, confirmed by tissue immunostaining, typical radiological findings and an appropriate clinical context. The first line treatment is the steroids with two warnings: Steroid resistance and relapse after discontinuation. In the case of steroid resistance, B cell depleting agents as rituximab represent the secondline treatment. In the case of relapse after discontinuation, steroid treatment may be associated with steroid sparing agents. Since the disease has been only recently identified, more prospective, long-term studies are needed to an improved understanding and a more correct and safe treatment.
文摘目的总结分析IgG4相关性疾病(immunoglobulin-G4 related disease,IgG4-RD)的妊娠及产后临床资料、诊治过程及随访结果,以提高产科医生对IgG4-RD的认识水平。方法采用回顾性研究方法对2023年1月18日北京大学深圳医院收治的1例(本例患者)IgG4相关甲状腺疾病成功妊娠的诊疗经过,以及通过数据库检索与妊娠相关的IgG4-RD病例报道进行分析。结果本例患者29岁,发现甲亢4年余、IgG4-RD 7个月、双胎妊娠,孕29周时因"甲亢危象、心功能Ⅳ级、IgG4-RD、子痫前期、新型冠状病毒感染等"于我院经多学科共同诊治,获得良好围产结局。文献检索到IgG4-RD合并妊娠病例报道6篇(共5例患者),包括本例患者在内的6例患者受累器官、临床表现不尽相同,经激素规范治疗后均获得良好围产结局。结论妊娠相关的IgG4-RD表现复杂多样,严重者可致重要脏器功能障碍。IgG4相关甲状腺疾病女性能够成功妊娠但存在较大风险,孕期及产后需持续激素维持治疗、多学科严密监测。
文摘患者男,77岁,间断腹泻2个月、发现免疫球蛋白升高1个月;既往无特殊病史。查体未见明显异常。实验室检查:血清IgMλ型M蛋白阳性,IgG417000.0mg/dl,IgM323.0mg/dl,便隐血阳性。因超声显示双侧颈部、腋窝多发淋巴结肿大而行^(18)F-FDG PET/CT显像,发现升结肠肝曲处软组织密度影,FDG摄取升高,最大标准摄取值(maximum standard uptake value,SUV_(max))为12.4(1A~1C).
